PEMPHIGUS VULGARIS

An autoimmune disease that produces bullous lesions on skin and mucous memgranes. Blisters are usually FLACCID and easily break and slough.

Caused by antibodies against desmoglein 3 (an epidermal glycoprotein)

Traditionally dx'd by detection of serum pemphigus Ab by imunofluorescence. ELISA available as of 1999, more specific (Arch. Derm. 135:143, 1999--AFP)

Treatment

1. Corticosteroids
2. Intravenous Immune Globulin
   1. In an open-label uncontrolled trial in 11 patients with severe pemphigus vulgaris unresponsive to conventional therapies including immune globulin treated with IV rituximab Qwk x 3wks followed by immune globulin therapy in the 4th week, repeated in cycles over 4 months, all patients had some response (NEJM 355:1772, 2006--JW)
   2. In a study in 61 pts with pemphigus vulgaris unresponsive to prednisolone randomized to IVIG 200-400mg/kg/d vs. placebo QD x 5d, there was a significant (and dose-dependent)  benefit with IVIG (J. Am. Acad. Dermatol. 60:595, 2009-JW)
3. Sulfasalazine + pentoxifylline
   1. In a study in 42 pts with moderate-to-severe pemphigus vulgaris randomized to sulfasalazine + pentoxifylline vs. placebo (all pts received systemic steroids + cyclophosphamide), at 8wks, incidence of "excellent" treatment response was 86% in the sulfasalazine/pentoxifylline group vs. 18% in the placebo group (Br. J. Dermatol. 161:313, 2009-JW)

In a study in 120 pts newly diagnosed with pemphigus vulgaris randomized to one of the following four treatments:
 1-Prednisolone 2mg/kg/day
 2-Prednisolone 2mg/kg/d + azathioprine 2.5mg/kg/d x 2mos, then 50mg/d
 3-Prednisolone 2mg/kg/d + mycophenolate mofetil
 4-Prednisolone 2mg/kg/d + (IV immune globulin 1g pulse therapy + cyclophosphamide) Qmo x 6mos then Q2mos x 6mos
All regimens were continued x 1y if tolerated.  #'s 2-4 all did  better than #1; #2 was had greatest benefit.  No sig. diff. between groups in adverse events (J. Am. Acad. Dermatol. 57:622, 2007--JW)